Malignant tumor of adrenal cortex without early diagnosis and radical surgical treatment is fatal. The results of treatment of 216 patients with adrenocortical carcinoma treated in 1968-2002, which is 32.6% of all the patients with tumors of adrenal cortex treated in the clinic were analyzed. In majority of cases the disease was hormonally active. The best result was observed after primary radical surgery at early stage (without regional metastases). Re-operation in majority of cases ensures remission, however, it cannot be considered as radical method of treatment. Chloditane application permits to prolongs survival, however it has usually been used as additional treatment.